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US "Atypical" Mad Cow Threat Was Predicted

The small scientific world of prion researchers -- the scientists who investigate "transmissible spongiform encephalopathies" (TSE) such as mad cow disease in cattle and Creutzfeldt-Jakob Disease (CJD) in humans -- is abuzz. That's because the two confirmed cases of US mad cow disease in Texas and Alabama are an "atypical" strain different from the British strain but identical to an atypical strain found so far in a small number of cattle in France, Germany, Poland and Sweden. The discovery of "atypical" mad cow disease in the US should not be surprising. Sheldon Rampton and I reported way back in 1997 that very strong evidence of an "atypical" TSE disease infecting US cattle was established by the work of Dr. Richard Marsh, the researcher to whom we dedicated our book Mad Cow USA.

Even before Britain confirmed its first case of mad cow, Dr. Marsh of the University of Wisconsin was investigating a similar disease in Stetsonville, Wisconsin, a 1985 outbreak in mink that he traced to Wisconsin dairy cattle. Marsh's published research confirmed suspicions among US scientists since the mid 1960s that the rare but deadly TSE disease in US mink -- transmissible mink encephalopathy or TME -- resulted from their having eaten TSE-infected US cattle.

Did the U.S. Infect Europe with Atypical Mad Cow Disease?

The discovery that the Texas and Alabama BSE cases are a variant strain identical to EU cases begs the question of whether the atypical EU cases resulted from European cattle being fed infected US feed made from rendered by-products and sold in Europe. After all, the US has been the biggest creator, user and exporter of by-product feed made from slaughterhouse waste. Also, scientists need to examine the TSE isolated by Richard Marsh in mink and traced to Wisconsin cattle, and compare it to the atypical BSE strain found in Texas, Alabama, France, Poland, Germany and Sweden. Is the Stetsonville TSE strain discovered by Richard Marsh the same strain as the US and EU atypical BSE cases, or is it another atypical strain?

Here below is our report on Marsh's discovery in 1985 of an atypical strain of BSE in US cattle from our 1997 book Mad Cow USA. (You can order the book for free from your favorite library and it is for sale in the usual places.)

Mad Cow USA

(The following excerpt is from Mad Cow USA, by Sheldon Rampton and John Stauber, pages 154-156:)

The common denominator in all of these [transmissible mink encephalopathy - TME] outbreaks was either "cattle" or "unknown." It was possible, of course, to imagine other scenarios, but Marsh believed he had at least strong circumstantial evidence that a TSE similar to mad cow disease already existed in U.S. cattle. "You can trace it back to feed real easy in mink," Marsh said. "And then you're left with the question, what was it in the feed that affected them? And what we find is it's these downer cows that are the common link. You don't have to be a genius to figure it out."

Within the field of veterinary medicine, "downer cow syndrome" was a "garbage can" category, used indiscriminately as the official diagnosis for any animal that died or had to be put down after failing to stand on its own legs for 24 hours or more. These included cows suffering from paralysis, arthritis, grass tetany, ketosis, bone fractures, and a form of calcium deficiency known as "milk fever." Most downer cows died from causes unrelated to the spongiform encephalopathies, but it was possible that the generic nature of the classification enabled some TSE-infected cows to slip into the mix.

It was impossible in practice to absolutely prove the link between downer cows and transmissible mink encephalopathy. By the time the disease appeared in mink, any cow that might have been the source would be long gone, its tissues unavailable for testing. To test his theory, therefore, Marsh did the next best thing—a series of experiments using brain matter from one of the mink that had died in the Stetsonville outbreak. He puréed the brain in a blender and used hypodermic syringes to inject the homogenized liquid into test animals: fourteen healthy mink, eight ferrets, two squirrel monkeys, twelve hamsters, forty-five mice and two Holstein bull calves.

The mice, remarkably, all stayed healthy, but every other species proved susceptible. The mink went down first, four months after inoculation. The two monkeys were the next to show neurological signs, at months nine and thirteen respectively. Two of the twelve hamsters survived, but the other ten succumbed in the fifteenth and sixteenth months. The two calves went down in months eighteen and nineteen. The ferrets lasted longest, but eventually the disease emerged in all but one of them, with incubation periods ranging between twenty-eight and thirty-eight months. These species barrier effects corresponded closely to the results from experiments with previous mink outbreaks.

Cattle are expensive test animals, and Marsh's experiments marked the first time that cattle had been tested for susceptibility to transmissible mink encephalopathy. His results proved that cattle could get the mink disease, and in turn led to unexpected new questions. "The real surprise of this experiment is that the clinical signs were quite different from what we've seen in Great Britain," he said. "This is what's changed our perspective on a surveillance of BSE in the United States. We thought BSE in the U.S. would look like BSE in Great Britain—a mad cow type of disease where the animal would have behavioral changes, become aggressive and look very much like a rabies infection does in cows."

Marsh's bull calves showed none of the unusual "mad" behavior that emerged as early warning signs in British cattle. "Eighteen months after inoculation, one animal simply collapsed in its holding room and could not be returned to a standing position," he reported. "This animal had shown no previous signs of behavioral change or loss of body condition. . . . The second animal was normal until nineteen months after inoculation when it too suddenly collapsed."

Indeed, the test bulls behaved exactly like downer cows—the type of animals which the Stetsonville rancher had been feeding to his mink. "The most disturbing finding of all is that they have very minimal spongiform lesions in their brains," Marsh said. In previous experiments with mink, he had shown that the spongy holes in brains were a secondary effect of the disease which did not always appear in noticeable quantities. Some mink breeds infected with TME would develop all of the usual clinical symptoms, but upon autopsy their brains showed a marked lack of spongiform degeneration. Now it appeared that cattle could also develop a form of TSE without the telltale lesions to aid in diagnosis. Their symptoms would look like downer cow syndrome, and even a brain autopsy might find nothing out of the ordinary.

"Without the brain lesions, the best way to diagnose the infection is a protein in the brain," Marsh said. "But there are only a few labs in the country that can look for this protein. This is not something that can be done by the local veterinarian or even most state diagnostic laboratories. You need to have pretty sophisticated means of testing. This is going to complicate our efforts at surveillance and testing for BSE in this country."

Histopathology and immunohistochemistry tests confirmed that Marsh's bulls had died of a spongiform encephalopathy, but it was a different strain of spongiform encephalopathy than the one that was killing cows in England. Its behavior in test animals showed significant differences also. In England, mice succumbed when exposed to brain tissue from mad cows, but hamsters seemed immune. In Marsh's experiments with the Stetsonville isolate of TME, the pattern was exactly the opposite: mice lived, but hamsters died.

From Mad Cows to Mad Mink and Back

To test whether passage through cattle altered the characteristics of the Stetsonville isolate, Marsh injected another 45 mice with brain tissue from his two test bulls. They also stayed healthy, just like the mice he had previously injected with mink brains. By itself, the fact that mink encephalopathy could infect cows was not terribly significant or surprising. After all, scientists had previously shown that TME could be transmitted to a wide variety of other test animals. What was significant was the result when Marsh took the brains of the dead bulls and used them on further tests with healthy mink. When backpassaged into mink, the bull brains behaved exactly the way mink brains behaved, causing symptoms of TME to emerge within four months after exposure by inoculation, or within seven months after oral exposure.

"There was no evidence for any deadaptation of the bovine agent for mink compared to . . . non-bovine-passaged mink brain," Marsh observed. "This suggests that there are no species barrier effects between mink and cattle in relation to the Stetsonville source of TME" — more evidence pointing to cattle as the source of the infection. "If mink on the Stetsonville ranch were exposed to TME by feeding them infected cattle, there must be an unrecognized scrapie-like disease of cattle in the United States," Marsh concluded. "If this is true, the disease is rare. The low incidence rate of TME and the fact that the Stetsonville mink rancher had fed products from fallen or sick cattle to his animals for the past 35 years suggests a very low prevalence of this disease."

The rarity of the disease, however, did not mean that it posed no danger. In fact, it could mean the very opposite. Mad cow disease had also been rare once in England. The very fact that it was rare, combined with its slow incubation period, were the factors that prevented the British from recognizing its dangers until it had already infected tens of thousands of animals. Moreover, the British had an advantage that U.S. farmers might not enjoy. Their strain of bovine spongiform encephalopathy was picked up fairly soon once cattle started behaving strangely. If a different strain of BSE existed in U.S. cattle— a strain where the animals didn't act deranged but simply fell over, like the cows in Marsh's tests—the disease could conceivably go unrecognized for a long time, invisible within the larger population of U.S. downer cows.

Every year, some 100,000 U.S. cows get classified as downers. Marsh was not suggesting that all 100,000 were carriers of a spongiform encephalopathy. What concerned him was the possibility that downer cow syndrome could mask the emergence of a TSE in the cattle population, allowing the disease to invisibly spread until it reached dangerous levels. It could multiply the same way it had multiplied in England, as rendering plants recycled the infection by converting sick animals into meat and bone meal which was then fed back to other cattle. The only certain way to prevent a cattle epidemic, therefore, would be to adopt the same policy that the British had already been forced to adopt: ban the practice of feeding rendered cows and other ruminant animals back to members of their own species.

### (End of excerpt)

How to Hide a Mad Cow

Today the ability to test cattle for mad cow disease has greatly advanced, and so-called rapid tests are used on all cattle before they are allowed into the human food chain in Japan, for example. I describe the situation in the United States as a cover-up of the extent of mad cow disease because the US needs to test millions of cattle a year, and in a transparent and verifiable way, before we can know with accuracy how much disease is present in the US herd. Currently the US is testing less than 1% of its cattle a year, and the procedures are shrouded in secrecy. The US forbids anyone but the government to conduct tests in the United States making it impossible for Americans to purchase meat that has been tested and found free of the disease.

In addition, despite the false PR assurances from government and the livestock industry, there is no "firewall feed ban" in the United States to completely stop the spread of mad cow disease. Today it is legal and widespread to feed US cattle on cattle fat contaminated with cattle protein, on cattle blood, and on poultry shit and litter contaminated with cattle protein. In addition, slaughterhouse waste from cattle is fed to pigs, and in turn the slaughterhouse waste from pigs is fed back to cattle.

We now know we have "atypical" mad cow disease in the US and even the USDA admits that it has probably been spreading for at least a decade through feeding cattle to cattle. Yet, the cannibal feeding practices continue and the US's mad cow testing program is a farce.

Dick Marsh died in 1997 before our book Mad Cow USA was published. He was a careful scientist who understood the precautionary principle and who worked tirelessly and was terribly and personally attacked for his prescient warnings that a unique strain of mad cow disease already existed in the US, and that unless the dangerous feeding practices of cow cannibalism were stopped, it would spread through cattle and threaten human health.

Perhaps if cancer had not silenced Dick Marsh a decade ago, his strong voice would have helped change the current dangerous policies of the United States Department of Agriculture (USDA) and the Food and Drug Administration (FDA). Currently these federal agencies are threatening animal and human safety in the US simply so the US government can protect and preserve the livestock industry's deadly but lucrative practice of animal cannibalism, turning slaughterhouse waste into cheap feed for cattle and other livestock.

##################### Bovine Spongiform Encephalopathy #####################
CJD WATCH MESSAGE BOARD
TSS
EXPORT of potential USDA CERTIFIED ATYPICAL AND TYPICAL TSE
Sat Jun 17, 2006 13:19
71.248.130.63
Greetings,
Thought some of you might be interested in the USDA exports of potential USDA CERTIFIED ATYPICAL AND TYPICAL MAD COW BRAINS, SWEETBREADS, BOVINE FROZEN OFFAL, AND LIVE CATTLE. Interestingly, the USA may be the one to blame from there consistent lies and deceit and what they have exported globally for decades, to blame for spreading sporadic CJD around the globe.
Looking from stats at ;
http://www.fas.usda.gov/ustrade/ustlists/ExCmdty.asp?QI=370619655344&type=1&code=02
then searching here ;
http://www.fas.usda.gov/ustrade/USTEXHS10.asp?QI=370619655344
SEEMS that Mexico received from the USA a boat load of potential mad cow brains 0206290030 between 2001 to 2005, Mexico received the most compared to COTE D'IVOIRE which was next in line, followed by ROMANIA, GREECE, SINGAPORE, GERMANY AND SWEDEN. ...
NEXT, looking at SWEETBREADS 0206290040 the USA exported, and whatever phenotype of TSE that went along, we have as follows;
MEXICO AGAIN receiving a boat load of sweetbreads, followed by ARGENTINA, JAPAN, URUGUAY, COLOMBIA, ISRAEL, BULGARIA, HONG KONG, VENEZUELA, United Arab Emirates, Switzerland, Singapore, Netherlands, The Bahamas, and the Dominican Republic. ...
THE LIST for BOVINE OFFAL FROZEN 020629 EXPORTED FROM THE USA ACROSS THE GLOBE IS PHENOMENAL WITH JAPAN RECEIVING THE MOST FROM 1998 TO 2003, FOLLOWED BY MEXICO, and from here the list is staggering along with the amount of potential TSE tainted materials. ...
FINALLY, LIVE CATTLE WITH CANADA RECEIVING THE MOST, FOLLOWED BY MEXICO, KOREA REPUBLIC OF, followed by many more countries with smaller amounts. ...
WHEN the OIE did away with the BSE GBR risk assessments to ride saddle with GW and his legal tool to trade TSE globally i.e. the BSE MRR policy, 20 years of fighting this disease went down the drain, just so he could trade his precious commodities and futures. THIS BSe about how now the USA having an epidemic of a spontaneous TSE in cattle and humans, as sporadic CJD triples in 3 years in the USA, is simply absurd. nothing is spontaneous about it, there is absolutely no science to back these 'spontaneous' statements up. ...
US "Atypical" Mad Cow Threat Was Predicted
https://www.prwatch.org/node/4883
TSS
#################### https://lists.aegee.org/bse-l.html ####################
##################### Bovine Spongiform Encephalopathy #####################
Subject: DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E. (PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES
Date: June 17, 2006 at 6:56 pm PST
Greetings list members,
here i go again. i must bring those mad sheep of mad river valley up again.
what about those mouse bio-assays? can one of the aphis/usda lurkers on this list, can one of them please comment please?
a declaration of emergency was announced ;
>> Imported
>> Belgium/Netherlands
>> Sheep Test Results
>> Background
>> Factsheet
>> Veterinary Services April 2002
>> APHIS
>
>
>
> snip...
>
>> Additional tests will be conducted to determine
>> exactly what TSE the animals haveBSE or scrapie.
>> These tests involve the use of bioassays that consist
>> of injecting mice with tissue from the infected animals
>> and waiting for them to develop disease. This testing
>> may take at least 2 to 3 years to complete.
>
>
>
> http://www.aphis.usda.gov/lpa/pubs/fsheet_faq_notice/fs_ahvtsheeptr.pdf
>
> DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E.
> (PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES
>
> http://frwebgate.access.gpo.gov/cgi-bin/getdoc.cgi?dbname=2000_register&docid=fr20jy00-31
>
>
> DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E
> (PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES [2]
>
> http://frwebgate.access.gpo.gov/cgi-bin/getdoc.cgi?dbname=2000_register&docid=fr20jy00-32
>
>
> or if those old urls dont work, go here;
>
> DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E
> (PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES
> - Terry S.
> Singeltary Sr. 7/20/00 (0)
>
> [Federal Register: July 20, 2000 (Volume 65, Number 140)] [Notices]
> [Page 45018] >From the Federal Register Online via GPO Access
> [wais.access.gpo.gov] [DOCID:fr20jy00-32]
>
> -----------------------------------------------------------------------
>
> DEPARTMENT OF AGRICULTURE
>
> Office of the Secretary
>
> [Docket No. 00-072-1]
>
> Declaration of Extraordinary Emergency Because of an Atypical
> Transmissible Spongiform Encephalopathy (Prion Disease) of Foreign Origin
>
> A transmissible spongiform encephalopathy (TSE) (prion disease) of
> foreign origin has been detected in the United States. It is different
> from TSE's previously diagnosed in the United States. The TSE was
> detected in the progeny of imported sheep. The imported sheep and
> their progeny are under quarantine in Vermont. Transmissible
> spongiform encephalopathies are degenerative fatal diseases that can
> affect livestock. TSE's are caused by similar, as yet uncharacterized,
> agents that usually produce spongiform changes in the brain.
> Post-mortem analysis has indicated positive results for an atypical
> TSE of foreign origin in four sheep in Vermont. Because of the
> potentially serious consequences of allowing the disease to spread to
> other livestock in the United States, it is necessary to seize and
> dispose of those flocks of sheep in Vermont that are affected with or
> exposed to the disease, and their germ plasm. The existence of the
> atypical TSE of foreign origin represents a threat to U.S. livestock.
> It constitutes a real danger to the national economy and a potential
> serious burden on interstate and foreign commerce. The Department has
> reviewed the measures being taken by Vermont to quarantine and
> regulate the flocks in question and has consulted with appropriate
> officials in the State of Vermont. Based on such review and
> consultation, the Department has determined that Vermont does not have
> the funds to compensate flock owners for the seizure and disposal of
> flocks affected with or exposed to the disease, and their germ plasm.
> Without such funds, it will be unlikely to achieve expeditious
> disposal of the flocks and germ plasm. Therefore, the Department has
> determined that an extraordinary emergency exists because of the
> existence of the atypical TSE in Vermont. This declaration of
> extraordinary emergency authorizes the Secretary to seize, quarantine,
> and dispose of, in such manner as he deems necessary, any animals that
> he finds are affected with or exposed to the disease in question, and
> their germ plasm, and otherwise to carry out the provisions and
> purposes of the Act of July 2, 1962 (21 U.S.C. 134-134h). The State of
> Vermont has been informed of these facts.
>
> Dated: This declaration of extraordinary emergency shall become
> effective July 14, 2000. Dan Glickman, Secretary of Agriculture. [FR
> Doc. 00-18367 Filed 7-19-00; 8:45 am] BILLING CODE 3410-34-P
>
> http://frwebgate.access.gpo.gov/cgi-bin/getdoc.cgi?dbname=2000_register&docid=fr20jy00-32
================================
> [Federal Register: July 20, 2000 (Volume 65, Number 140)] [Notices]
> [Page 45018] >From the Federal Register Online via GPO Access
> [wais.access.gpo.gov] [DOCID:fr20jy00-31]
>
> ========================================================================
> Notices Federal Register
> ________________________________________________________________________
>
> This section of the FEDERAL REGISTER contains documents other than
> rules or proposed rules that are applicable to the public. Notices of
> hearings and investigations, committee meetings, agency decisions and
> rulings, delegations of authority, filing of petitions and
> applications and agency statements of organization and functions are
> examples of documents appearing in this section.
>
> ========================================================================
>
> [[Page 45018]]
>
> -----------------------------------------------------------------------
>
> DEPARTMENT OF AGRICULTURE
>
> Office of the Secretary
>
> [Docket No. 00-072-2]
>
> Declaration of Emergency Because of an Atypical Transmissible
> Spongiform Encephalopathy (Prion Disease) of Foreign Origin
>
> A transmissible spongiform encephalopathy (TSE) (prion disease) of
> foreign origin has been detected in the United States. It is different
> from TSE's previously diagnosed in the United States. The TSE was
> detected in the progeny of imported sheep. The imported sheep and
> their progeny are under quarantine in Vermont. Transmissible
> spongiform encephalopathies are degenerative fatal diseases that can
> affect livestock. TSE's are caused by similar, as yet uncharacterized,
> agents that usually produce spongiform changes in the brain.
> Post-mortem analysis has indicated positive results for an atypical
> TSE of foreign origin in four sheep in Vermont. Because of the
> potentially serious consequences of allowing the disease to spread to
> other livestock in the United States, it is necessary to seize and
> dispose of those flocks of sheep in Vermont that are affected with or
> exposed to the disease, and their germ plasm. The existence of the
> atypical TSE of foreign origin represents a threat to U.S. livestock.
> It constitutes a real danger to the national economy and a potential
> serious burden on interstate and foreign commerce. APHIS has
> insufficient funds to carry out the seizure and disposal of animals
> and germ plasm necessary to eliminate this disease risk. These funds
> would be used to compensate the owners of the animals and germ plasm
> for their seizure and disposal in accordance with 21 U.S.C. 134a.
> Therefore, in accordance with the provisions of the Act of September
> 25, 1981, as amended (7 U.S.C. 147b), I declare that there is an
> emergency that threatens the livestock industry of this country and
> hereby authorize the transfer and use of such funds as may be
> necessary from appropriations or other funds available to agencies or
> corporations of the United States Department of Agriculture to seize
> and dispose of animals that are affected with or exposed to this TSE,
> and their germplasm, in accordance with 21 U.S.C. 134a.
>
> Dated: This declaration of emergency shall become effective July 14,
> 2000. Dan Glickman, Secretary of Agriculture. [FR Doc. 00-18368 Filed
> 7-19-00; 8:45 am] BILLING CODE 3410-34-P
>
> I was told that ;
>
>
> -------- Original Message --------
> Subject: Re: hello Dr. Sutton...question please...scrapie...TSS
> Date: Thu, 20 May 2004 14:36:09 -0400
> From: Jim.D.Rogers@aphis.usda.gov
> To: flounder@wt.net
snip...
FULL TEXT AND THREAD BETWEEN TSS, MAFF, USDA AND DR. DETWILER HERE ;
https://web01.aphis.usda.gov/regpublic.nsf/168556f5aa7a82ba85256ed00044eb1f/eff9eff1f7c5cf2b87256ecf000df08d?OpenDocument
Greetings again BSE-L members,
NOW, i cannot for the life of me figure out why we have not heard anything about those mouse bio-assays of those mad sheep of mad river valley, and atypical TSE ? i mean hell, there was a DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E (PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES and we never hear of final results, is this not another case of the TEXAS BSE PROTOCOLS of just never confirming anything unless the GAO gets involved? maybe USDA could comment on this now? or is this too like those WMD, just something that never existed? i know Dr. Detwiler is out of the loop on this now, but there are others here that could answer this question if they wanted too and or could???
QUOTE ;
1998
Dr. Detwiler replied. "There is new research which shows that sheep can contract BSE" ......"information I can't divulge".....end
WHY, after some 7 years, do we still not have any answers ???
WHERE are those mouse bio-assays ???
PLEASE look on every shelf, maybe same one that those TEXAS MAD COW tissue samples were left on for 7+ months before finally confirming after a Congressional order and or end around, they could be there. ...
still disgusted in sunny Bacliff, Texas
Terry S. Singeltary Sr.
FSA 06/06/03 AGENDA 3.1, 15 JUNE 2006
ATYPICAL SCRAPIE IN SMALL RUMINANTS: CONSIDERATION OF THE
CURRENT PRECAUTIONARY RISK MANAGEMENT MEASURES
Executive Summary
1. This paper provides information on atypical scrapie (a transmissible spongiform
encephalopathy (TSE)) in sheep and goats and the precautionary measures
currently in place to protect consumers from the possible risks from TSEs in
these species. There are a great many unknowns about atypical scrapie,
including the potential implications, if any, for human health.
2. It also reports on the views of stakeholders and consumer focus groups who
were asked whether, in the light of this uncertainty, additional precautionary
measures were needed and for their views on the Agency’s advice on this
subject.
3. The Board is asked to:
• note that the Agency’s advice has been reworded to take account of the views
of stakeholders and the consumer focus groups and will be tested further
• note that the background information on sheep TSEs on the Agency’s website
will be reviewed
• note that the agricultural departments are planning to review the Ram
Genotyping Scheme
• note that surveillance for atypical scrapie will be maintained in order to detect
any changes in prevalence.
• agree that the Agency’s advice and recommendations on precautionary
measures should be kept under review and be brought back to the Board if
there are significant changes in the understanding of the risk.
• agree that developments on atypical scrapie be kept under review to enable
contingency policy to be refined as new information emerges.
• agree that the Agency should open discussions with the European
Commission on the issue of the identification of meat from older sheep or
goats and natural sausage casings made from sheep intestines to enable
consumer choice.
2
TSE DIVISION
Contacts:
Alison Gleadle Tel: 020 7276 8303
Email: alison.gleadle@foodstandards.gsi.gov.uk
Irene Hill Tel: 020 7276 8324
Email: irene.hill@foodstandards.gsi.gov.uk
3
FSA 06/06/03 AGENDA ITEM 3.1, 15 JUNE 2006
ATYPICAL SCRAPIE IN SHEEP AND GOATS: CONSIDERATION OF THE
CURRENT PRECAUTIONARY RISK MANAGEMENT MEASURES
Issue
1. To consider whether the Agency should recommend, on the basis of current
evidence, that additional precautionary measures are needed to reduce the
possible risk to consumers from atypical scrapie.......
snip...
Conclusions
27. Atypical scrapie is definitely present in the UK flock, and in the flocks of other
Member States (MS), and animals with atypical scrapie have, and will be,
entering the food supply. However it is not known if this constitutes any risk to
human health. Unlike the situation when BSE was first discovered in cattle,
precautionary measures are already in place. Based on the limited knowledge of
the distribution of infectivity in atypical scrapie, the SEAC Subgroup concluded
that the SRM requirements that were put in place on a precautionary basis for
BSE in sheep may provide at least a similar level of protection against the
possible risk from atypical scrapie.
28. The consideration of the proportionality of any additional precautionary measures
is very difficult when the human health risk is unknown, and, as reported by
SEAC, there is insufficient data to carry out a risk assessment.
29. Any additional precautionary measures that could be put in place have a high
economic cost, are currently highly impractical (see Annex 1 for details) and
would impose a cost on industry that would, according to industry stakeholders,
be likely to bring into question the economic viability of sheep farming. ...
snip...
full text ;
http://www.food.gov.uk/multimedia/pdfs/fsa060603.pdf
FSA 06/06/04 AGENDA ITEM 3.2, 15 JUNE 2006
BSE AND SHEEP CONTINGENCY POLICY
Executive Summary
1. This paper asks the Board to agree, for purposes of contingency planning, a
possible approach to a graduated strengthening of measures to protect
consumers in response to one or more findings of BSE in the current UK sheep
flock.
2. The paper also notes the high level of uncertainty around estimates of the
possible risk from BSE in sheep and that, if BSE were ever found in a UK sheep,
the estimate of the risk to consumers would depend on the accumulated results
of surveillance for BSE in sheep up to that time. It therefore recommends that the
policy be kept under review and that any policy agreed now on a contingency
basis should urgently be reconfirmed taking into account the circumstances at the
time of any finding of BSE in a UK sheep.
3. The Board is invited to:
• note that, in the event of confirmation of BSE in a sheep, targeted testing of
animals in the affected flock or flocks would be carried out to assist in
determining the potential spread of the disease and whether it may have
entered the food supply (paragraph 9).
• agree that an expert group be set up to advise on what additional surveillance
should be put in place, if BSE were to be found in a UK sheep, to improve
estimates of prevalence of BSE in UK sheep (paragraph 13).
• agree that, on current knowledge, it would advise the following graduated
response to one or more findings of BSE in the current UK sheep flock:
• one finding of BSE in sheep - remove additional SRM;
• two findings of BSE in unrelated flocks - exclude sheep aged over 12
months from the food supply and remove the additional SRM from the
remaining sheep;
• three findings of BSE in unrelated flocks - allow into the food supply only
sheep that were either genetically resistant to BSE or semi-resistant and
aged under 12 months and remove the additional SRM from those sheep
(paragraph 20).
2
• agree that its contingency policy for a finding of BSE in sheep should be kept
under review and be urgently reconfirmed should BSE actually be found in a
UK sheep (paragraph 22).
• comment on the outline handling plan at Annex F and the strategy for the
external communication that would be needed (paragraph 30).
TSE Division
Contacts:
Alison Gleadle Tel: 020 7276 8303 (GTN 7276 8303)
Email: alison.gleadle@foodstandards.gsi.gov.uk
David Carruthers Tel: 020 7276 8305 (GTN 7276 8305)
Email: david.carruthers@foodstandards.gsi.gov.uk
snip...
http://www.food.gov.uk/multimedia/pdfs/fsa060604.pdf
Subject: REPORT OF THE COMMITTEE ON SCRAPIE November 9, 2005 USAHA
Date: February 12, 2006 at 1:03 pm PST
REPORT OF THE COMMITTEE ON SCRAPIE
Chair: Dr. Jim Logan, Cheyenne, WY
Vice Chair: Dr. Joe D. Ross, Sonora, TX
Dr. Deborah L. Brennan, MS; Dr. Beth Carlson, ND; Dr. John R. Clifford, DC; Dr. Thomas F. Conner, OH; Dr. Walter E. Cook, WY; Dr. Wayne E. Cunningham, CO; Dr. Jerry W. Diemer, TX; Dr. Anita J. Edmondson, CA; Dr. Dee Ellis, TX; Dr. Lisa A. Ferguson, MD; Dr. Keith R. Forbes, NY; Dr. R. David Glauer, OH; Dr. James R. Grady, CO; Dr. William L. Hartmann, MN; Dr. Carolyn Inch, CAN; Dr. Susan J. Keller, ND; Dr. Allen M. Knowles, TN; Dr. Thomas F. Linfield, MT; Dr. Michael R. Marshall, UT; Dr. Cheryl A. Miller, In; Dr. Brian V. Noland, CO; Dr. Charles Palmer, CA; Dr. Kristine R. Petrini, MN; Mr. Stan Potratz, IA; Mr. Paul E. Rodgers, CO; Dr. Joan D. Rowe, CA; Dr. Pamela L. Smith, IA; Dr. Diane L. Sutton, MD; Dr. Lynn Anne Tesar, SD; Dr. Delwin D. Wilmot, NE; Dr. Nora E. Wineland, CO; Dr. Cindy B. Wolf, MN.
The Committee met on November 9, 2005, from 8:00am until 11:55am, Hershey Lodge and Convention Center, Hershey, Pennsylvania. The meeting was called to order by Dr. Jim Logan, chair, with vice chairman Dr. Joe D. Ross attending. There were 74 people in attendance.
The Scrapie Program Update was provided by Dr. Diane Sutton, National Scrapie Program Coordinator, United States Department of Agriculture (USDA), Animal and Plant Health Inspection Services (APHIS), Veterinary Services (VS). The complete text of the Status Report is included in these Proceedings.
Dr. Patricia Meinhardt, USDA-APHIS-VS-National Veterinary Services Laboratory (NVSL) gave the Update on Genotyping Labs and Discrepancies in Results. NVSL conducts investigations into discrepancies on genotype testing results associated with the Scrapie Eradication Program. It is the policy of the Program to conduct a second genotype test at a second laboratory on certain individual animals. Occasionally, there are discrepancies in those results. The NVSL conducts follow-up on these situations through additional testing on additional samples from the field and archive samples from the testing laboratories.
For the period of time from January 1, 2005, until October 15, 2005, there were 23 instances of discrepancies in results from 35 flocks. Of those 23 instances, 14 were caused by laboratory error (paperwork or sample mix-up), 3 results from field error, 5 were not completely resolved, and 1 originated from the use of a non-approved laboratory for the first test. As a result of inconsistencies, one laboratory’s certification was revoked by APHIS-VS.
To reduce/eliminate these problems, the Program has placed additional quality requirements on the testing laboratories: additional review of final reports, additional coding systems for testing operations, strict follow-up and reports to NVSL on corrective actions, dual data entry systems, and more frequent inspections.
The Agricultural Research Services (ARS) Scrapie Research Update was given by Janet Alverson, USDA- ARS. Dr. Alverson reported on the effect of multiple births and fetal position within the uterus on PrP-Sc accumulation in fetal cotyledons. Fetal cotyledons of fetuses with
resistant genotypes can accumulate PrP-Sc when positioned next to a fetus of susceptible genotype with cotyledons positive for PrP-Sc accumulation.
Scrapie Surveillance Evaluation Working Group Update was presented by Tracey Lynn, Epidemiologist with the National Surveillance Unit, Center for Epidemiology and Animal Health (CEAH). The presentation provided a background on evaluation, a quick review of analyses completed to date by the scrapie surveillance evaluation working group, and some of the preliminary findings. The process of surveillance system evaluation is undertaken to assist a disease control program with identifying possible improvements to their surveillance system, and includes an assessment of the overall utility of the system, identification of potential gaps in coverage, and an evaluation of the overall performance of the system. The scrapie surveillance evaluation working group reviewed the structure and processes of the scrapie surveillance program, as well as various quality and effectiveness measures.
Overall, 98-99% of surveillance samples come from the Regulatory Scrapie Surveillance System (RSSS), so the RSSS system has been the primary focus of the evaluation process. The working group developed a flow chart indicating the flow of sheep through RSSS, which identified potential gaps in surveillance coverage, including custom kill plants and sheep being exported to Mexico. Spatial analyses can assist in identifying areas with high density sheep populations with lower levels of RSSS sampling. Identification compliance is being evaluated by reviewing reports from slaughter plants on the proportion of animals with appropriate identification. Additional analyses remain, including defining the most appropriate economic analyses, and comparing the surveillance system with developing surveillance standards. The working group hopes to have a draft written report for review by the end of the year.
Giving the Update on Scrapie Diagnostics and Susceptibility was Katherine O’Roarke, Research Microbiologist, USDA-ARS. "What’s New in Scrapie" -- Biopsy sampling of the third eyelid or tonsillar lymphoid tissue is a useful live animal test for scrapie. The biopsy sample is examined for accumulation of the abnormal prion protein using immunohistochemistry. A joint project conducted by the Veterinary Laboratory Agencies and the Moredun Institute in the United Kingdom has developed an alternative technique in which tissue is collected from the narrow band of lymphoid tissue near the rectal-anal junction. The morphology of the lymphoid follicles is similar in the tonsil, retropharyngeal lymph nodes, third eyelid, and rectal-anal mucosal tissue. A report on more than 300 sheep in the United Kingdom (UK), prepared by Drs. Lorenzo Gonzalez and Jeffrey Martin, will describe the sensitivity, specificity, and optimal collection interval for this technique in a variety of breeds of British sheep. ARS has done a preliminary evaluation of the technique in US sheep. Samples of third eyelid and rectal-mucosal tissue were collected from 56 sheep. Forty-two (42) sheep had negative biopsies at both sites; most of these sheep have been necropsied and no PrP-d was found in retropharyngeal lymph node or tonsil, showing good agreement with the antemortem biopsies. Fourteen (14) sheep had positive rectal biopsy samples; of those, only 12 had positive eyelid biopsies. These sheep will be monitored for disease development. However, the protocol is identical for all samples and it is probable that these sheep represent false negative third eyelid results. Abstracts of reports on the UK studies indicate that sensitivity of the test was 70% in the UK; similar large scale testing on US sheep is necessary. The biopsy tissue is somewhat difficult to handle in the tissue processing laboratory and adaptation to an ELISA format may improve test performance.
Alexia McKnight, Assistant Professor of Radiology, University of Pennsylvania, reviewed magnetic resonance imaging (MRI) diagnostics before the committee. A synopsis containing references is attached at the end of this report. Dr. McKnight asked the question, "could MRI be a cost-effective screening test, estimated at $25-30 each with results immediately available." The committee feels that it is not practical as compared to other alternatives currently available. However, the committee expressed interest in future reference to this technology.
Dr. Diane Sutton lead the Uniform Methods and Rules (UM&R) and Regulatory Issues Discussion. Several modifications to the UM&R were discussed. Eight issues were identified and communicated to the APHIS scrapie program coordinator. The committee acknowledged that APHIS and the industry is adequately addressing the year-to-year industry concerns.
Dr. Kris Petrini representing the North Central United States Animal Health Association District presented five recommendations to the Committee. During the discussions regarding these recommendations it was evident that all five issues had been addressed during the year at this Committee meeting.
The Committee approved a recommendation that USDA-APHIS-VS continue to provide indemnity funds for animals that have been designated for testing in Flocks Under Investigation as an alternative to third eyelid testing after consultation with the designated Scrapie Epidemiologist (DSE) and the Regional Area Epidemiologist (RAE).
The 2004 Resolutions along with their responses were reviewed by the Committee.
A Resolution concerning premises registration and identification was approved by the Committee and forwarded to the Committee on Nominations and Resolutions.
Committee on Scrapie
Status Report-Fiscal Year 2005: Cooperative State-Federal Scrapie Eradication Program
Submitted by Diane Sutton, DVM and Gary Ross, DVM
National Center for Animal Health Programs, APHIS, USDA
In Fiscal Year 2005 the Scrapie Eradication Program focused on: (1) utilization of a genetic based approach to flock clean-up plans; (2) cleaning up infected and source flocks; (3) tracing and testing exposed animals and flocks; (4) expansion of regulatory slaughter surveillance (RSSS); (5) conducting considtent state reviews, (6) producer education; (7) upgrading of the Scrapie National Generic Database and (8) publishing the updated Scrapie Eradication Uniform Methods and Rules (UM&R). The current Scrapie Eradication UM&R is posted at http://www.aphis.usda.gov/vs/nahps/scrapie/umr-scrapie-erad.pdf.
Consistent State Reviews
States must meet the requirements in 9 CFR 79.6 in order to move sheep and goats in interstate commerce with minimal restrictions. Twenty seven states have enacted the required identification rules, the remaining states have submitted a work plan that describes the steps that will be taken to comply and provided a timeline for completing significant milestones. USDA is conducting onsite scrapie program consistent state reviews and has completed reviews in 12 states. States must be in full compliance by the end of their current rule making cycle. States not in full compliance at that time will be removed from the consistent state list. Removal from the list would create a significant impact on the interstate movement of sheep and goats from those States.
Scrapie Flock Certification Program
As of September 30, 2005, there were 1,961 flocks participating in the Scrapie Flock Certification Program (SFCP). Of these flocks 188 were certified flocks, 1,770 were complete monitored flocks, and 3 were selective monitored flocks (figure 2). There were 209 flocks newly enrolled and 53 newly certified (13 with status dates in FY 2005 and 40 with status dates in previous years) in FY 2005 (figure 3).
Infected and Source Flocks
As of September 30, 2005, there were 105 scrapie infected and source flocks. There were a total of 165** new infected and source flocks reported for FY 2005. The total infected and source flocks that have been released in FY 2005 was 128. The ratio of infected and source flocks cleaned up or placed on clean up plans vs. new infected and source flocks discovered in FY 2005 was 1.03 : 1*. In addition 622 scrapie cases were confirmed and reported by the National Veterinary Services Laboratories (NVSL) in FY 2005, of which 130 were RSSS cases. Fifteen cases of scrapie in goats have been reported since 1990. The last goat case was reported in May 2005. Approximately 5,626 animals were indemnified comprised of 49% non-registered sheep, 45% registered sheep, 1.4% non-registered goats and 4.6% registered goats.
Regulatory Scrapie Slaughter Surveillance (RSSS)
RSSS was designed to utilize the findings of the Center for Epidemiology and Animal Health (CEAH) Scrapie: Ovine Slaughter Surveillance (SOSS) study. The results of SOSS can be found at http://www.aphis.usda.gov/vs/ceah/cahm/Sheep/sheep.htm . RSSS started April 1,
2003. It is a targeted slaughter surveillance program which is designed to identify infected flocks for clean-up. During FY 2005 collections increased by 32% overall and by 90% for black and mottled faced sheep improving overall program effectiveness and efficiency as demonstrated by the 26% decrease in percent positive black faced sheep compared to FY 2004. Samples have been collected from 62,864 sheep since April 1, 2003, of which results have been reported for 59,105 of which 209 were confirmed positive. During FY 2005, 33,137 samples were collected from 81 plants. There have been 130 NVSL confirmed positive cases (30 collected in FY 2004 and confirmed in FY 2005 and 100 collected and confirmed in FY 2005) in FY 2005. Face colors of these positives were 114 black, 14 mottled, 1 white and 1 unknown. The percent positive by face color is shown in the chart below.
Scrapie Testing
In FY 2005, 35,845 animals have been tested for scrapie: 30,192 RSSS; 4,742 regulatory field cases; 772 regulatory third eyelid biopsies; 10 third eyelid validations; and 129 necropsy validations (chart 9).
Animal ID
As of October 04, 2005, 103,580 sheep and goat premises have been assigned identification numbers in the Scrapie National Generic Database. Official eartags have been issued to 73,807 of these premises.
*This number based on an adjusted 12 month interval to accommodate the 60 day period for setting up flock plans.
http://www.usaha.org/committees/reports/2005/report-scr-2005.pdf
Subject: SCRAPIE USA UPDATE AS of March 31, 2006 2 NEW CASES IN GOAT, 82
INFECTED SOURCE FLOCKS, 19 INFECTED RSSS
Date: April 30, 2006 at 4:49 pm PST
SCRAPIE USA UPDATE AS of March 31, 2006
2 NEW CASES IN GOAT, 82 INFECTED SOURCE FLOCKS, WITH 4 NEW INFECTED SOURCE
FLOCKS IN MARCH, WITH 19 SCRAPIE INFECTED RSSS REPORTED BY NVSL
http://www.aphis.usda.gov/vs/nahps/scrapie/monthly_report/monthly-report.html
12/10/76
AGRICULTURAL RESEARCH COUNCIL
REPORT OF THE ADVISORY COMMITTE ON SCRAPIE
Office Note
CHAIRMAN: PROFESSOR PETER WILDY
snip...
A The Present Position with respect to Scrapie
A] The Problem
Scrapie is a natural disease of sheep and goats. It is a slow
and inexorably progressive degenerative disorder of the nervous system
and it ia fatal. It is enzootic in the United Kingdom but not in all
countries.
The field problem has been reviewed by a MAFF working group
(ARC 35/77). It is difficult to assess the incidence in Britain for
a variety of reasons but the disease causes serious financial loss;
it is estimated that it cost Swaledale breeders alone $l.7 M during
the five years 1971-1975. A further inestimable loss arises from the
closure of certain export markets, in particular those of the United
States, to British sheep.
It is clear that scrapie in sheep is important commercially and
for that reason alone effective measures to control it should be
devised as quickly as possible.
Recently the question has again been brought up as to whether
scrapie is transmissible to man. This has followed reports that the
disease has been transmitted to primates. One particularly lurid
speculation (Gajdusek 1977) conjectures that the agents of scrapie,
kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of
mink are varieties of a single "virus". The U.S. Department of
Agriculture concluded that it could "no longer justify or permit
scrapie-blood line and scrapie-exposed sheep and goats to be processed
for human or animal food at slaughter or rendering plants" (ARC 84/77)"
The problem is emphasised by the finding that some strains of scrapie
produce lesions identical to the once which characterise the human
dementias"
Whether true or not. the hypothesis that these agents might be
transmissible to man raises two considerations. First, the safety
of laboratory personnel requires prompt attention. Second, action
such as the "scorched meat" policy of USDA makes the solution of the
acrapie problem urgent if the sheep industry is not to suffer
grievously.
snip...
76/10.12/4.6
http://www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf
Published online before print October 20, 2005
Proc. Natl. Acad. Sci. USA, 10.1073/pnas.0502296102
Medical Sciences
A newly identified type of scrapie agent can naturally infect sheep with resistant PrP genotypes
( sheep prion | transgenic mice )
Annick Le Dur *, Vincent Béringue *, Olivier Andréoletti , Fabienne Reine *, Thanh Lan Laï *, Thierry Baron , Bjørn Bratberg ¶, Jean-Luc Vilotte ||, Pierre Sarradin **, Sylvie L. Benestad ¶, and Hubert Laude *
*Virologie Immunologie Moléculaires and ||Génétique Biochimique et Cytogénétique, Institut National de la Recherche Agronomique, 78350 Jouy-en-Josas, France; Unité Mixte de Recherche, Institut National de la Recherche Agronomique-Ecole Nationale Vétérinaire de Toulouse, Interactions Hôte Agent Pathogène, 31066 Toulouse, France; Agence Française de Sécurité Sanitaire des Aliments, Unité Agents Transmissibles Non Conventionnels, 69364 Lyon, France; **Pathologie Infectieuse et Immunologie, Institut National de la Recherche Agronomique, 37380 Nouzilly, France; and ¶Department of Pathology, National Veterinary Institute, 0033 Oslo, Norway
Edited by Stanley B. Prusiner, University of California, San Francisco, CA, and approved September 12, 2005 (received for review March 21, 2005)
Scrapie in small ruminants belongs to transmissible spongiform encephalopathies (TSEs), or prion diseases, a family of fatal neurodegenerative disorders that affect humans and animals and can transmit within and between species by ingestion or inoculation. Conversion of the host-encoded prion protein (PrP), normal cellular PrP (PrPc), into a misfolded form, abnormal PrP (PrPSc), plays a key role in TSE transmission and pathogenesis. The intensified surveillance of scrapie in the European Union, together with the improvement of PrPSc detection techniques, has led to the discovery of a growing number of so-called atypical scrapie cases. These include clinical Nor98 cases first identified in Norwegian sheep on the basis of unusual pathological and PrPSc molecular features and "cases" that produced discordant responses in the rapid tests currently applied to the large-scale random screening of slaughtered or fallen animals. Worryingly, a substantial proportion of such cases involved sheep with PrP genotypes known until now to confer natural resistance to conventional scrapie. Here we report that both Nor98 and discordant cases, including three sheep homozygous for the resistant PrPARR allele (A136R154R171), efficiently transmitted the disease to transgenic mice expressing ovine PrP, and that they shared unique biological and biochemical features upon propagation in mice. These observations support the view that a truly infectious TSE agent, unrecognized until recently, infects sheep and goat flocks and may have important implications in terms of scrapie control and public health.
--------------------------------------------------------------------------------
Author contributions: H.L. designed research; A.L.D., V.B., O.A., F.R., T.L.L., J.-L.V., and H.L. performed research; T.B., B.B., P.S., and S.L.B. contributed new reagents/analytic tools; V.B., O.A., and H.L. analyzed data; and H.L. wrote the paper.
A.L.D. and V.B. contributed equally to this work.
To whom correspondence should be addressed.
Hubert Laude, E-mail: laude@jouy.inra.fr
www.pnas.org/cgi/doi/10.1073/pnas.0502296102
http://www.pnas.org/cgi/content/abstract/0502296102v1
Of greatest interest today is the BSE agent because it is the presumptive cause of new variant CJD and must be considered a demonstrated risk to human health. The scrapie agent poses a theoretical risk to human health.
Today we ask you to consider the implications of two theoretical possibilities: the first, that sheep and goats in BSE countries theoretically might be infected with the BSE agent, and Professor Almond, who headed a subcommittee of the United Kingdom's Spongiform Encephalopathy Advisory Committee, has agreed to review that topic for us today.
Then scrapie, which theoretically might be a human pathogen, though there's no hard evidence for that, and of course, some number of sheep and goats in many countries, including the United States, are infected with the scrapie agent.
Now, let me say now that no U.S. government regulatory authority would ever knowingly permit humans or animals to be exposed to a product containing the scrapie agent, but considering the nature of the scrapie agent and the disease, we are not so naive as to think that such exposures have not already occurred. ...
FULL TEXT ;
http://www.fda.gov/ohrms/dockets/ac/99/transcpt/3518t2.rtf
http://72.14.209.104/searchq=cache:pKJPlLI2R44J:www.fda.gov/ohrms/dockets/ac/99/transcpt/3518t2.rtf+scrapie+strains+breed+east+friesian&hl=en&gl=us&ct=clnk&cd=23
TSS
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##################### Bovine Spongiform Encephalopathy #####################
Subject: PrP(d) accumulation in organs of ARQ/ARQ sheep experimentally infected with BSE by peripheral routes
Date: June 19, 2006 at 8:11 am PST
PrP(d) accumulation in organs of ARQ/ARQ sheep experimentally infected with BSE by peripheral routes.
Lezmi S, Ronzon F, Bencsik A, Bedin A, Calavas D, Richard Y, Simon S, Grassi J, Baron T.
Agence Francaise de Securite Sanitaire des Aliments (AFSSA), Lyon cedex 07, France.
To study the pathogenesis of bovine spongiform encephalopathy infection in small ruminants, two Lacaune sheep with the AA136RR154QQ171 and one with the AA136RR154RR171 genotype for the prion protein, were inoculated with a brain homogenate from a French cattle BSE case by peripheral routes. Sheep with the ARQ/ARQ genotype are considered as susceptible to prion diseases contrary to those with the ARR/ARR genotype. The accumulation of disease-associated prion protein (PrP(d)) was analysed by biochemical and immunohistochemical methods. No PrP(d) accumulation was detected in samples from the ARR/ARR sheep 2 years post inoculation. In the two ARQ/ARQ sheep that had scrapie-like clinical symptoms, PrP(d) was found in the central, sympathetic and enteric nervous systems and in lymphoid organs. Remarkably, PrP(d) was also detected in some muscle types as well as in all peripheral nerves that had not been reported previously thus revealing a widespread distribution of BSE-associated PrP(d) in sheep tissues.
snip...
Results and Discussion
For all samples analysed from the ARR/ARR sheep, no PrPd was detected by any of the three
PrPd detection methods used (table 1). This result correlates with the higher genetic resistance
to TSE associated with this genotype naturally affected with scrapie (Elsen et al., 1999) or
orally infected with the BSE agent (Jeffrey et al., 2001). However, resistance of the
ARR/ARR sheep challenged with TSE infection is not considered complete since natural
scrapie cases have been reported in sheep with this genotype (Buschmann et al., 2004); (Ikeda
et al., 1995) (French surveillance program; unpublished data). Furthermore, BSE has been
transmitted to ARR/ARR sheep by the intra-cerebral route (Houston et al., 2003b).
In both ARQ/ARQ sheep, the CNS (including retina), the lymphoid system and the
autonomous nervous system were identified by each method as major sites of PrPd
accumulation (table 1 ; figure 1) and were also described earlier by other groups in
experimentally BSE affected sheep (Foster et al., 2001; Jeffrey et al., 2001) as well as in
naturally scrapie-affected sheep (Jeffrey et al., 2001; van Keulen et al., 1999). In the CNS, the
quantities of abnormal PrP, expressed as equivalent in recPrP, were estimated by ELISA at up
to 13000 ng/g of brainstem tissue. Comparatively, the levels found in 13 ARQ/ARQ or
ARQ/VRQ sheep clinically affected with natural scrapie averaged 40 000 ± 20 000 ng of
PrPd/g of CNS tissues. Lymphoid organs accumulated lower levels of PrPd and large
quantities of material were required to detect a signal by WB in the mandibular or iliac medial
Copyright @ Acta Biochimica Polonica, Paper in Press, No. 1273
- 5 -
lymph nodes (LN) of SB3 (Figure 1). In the spleen of SB1 and SB3, 46 and 2 ng equivalent of
PrPd/g of tissue were detected, respectively. In the ileum, 232 ng equivalent of PrPd /g of
tissue was detected and correlated with a higher number and size of germinal centres when
compared to spleen or iliac LN. The mean quantity of PrPd in the CNS was 187 and 36 fold
greater than the quantities determined respectively in spleen and in the intestine.
Qualitatively, different types of PrPd deposits in the brain were identified from the frontal
cortex to the lumbar spinal cord. These PrPd deposits were mainly identical to those
previously identified in scrapie- or BSE-affected sheep (Gonzalez et al., 2002; Ryder et al.,
2001). In the retina, PrPd accumulation was mainly detected in the ganglionar layer (1), intern
(2) and extern (4) plexiform layers (numbers corresponding to the different layers in the
retina, figure 2a). Interestingly, in the enteric nervous system of ARQ/ARQ sheep, PrPd was
detected associated with neurons (figure 2g) as well as in the coeliac ganglia in which intraand
peri-neuronal PrPd deposits were visualized (figure 2h). In the adrenal gland, two types of
PrPd accumulation were observed as dense intracellular or synaptic-like deposits (figure 2i).
In lymphoid organs, PrPd was detected in germinal centres of secondary lymphoid follicles,
in follicular dendritic cells and in tingible body macrophages (figure 2j). PrPd was also
detected in cells with a morphology consistent with macrophages in the subcapsular sinus of
some lymph nodes (figure 2j, arrowhead, table 1*). These observations are in agreement with
previous results obtained both in sheep naturally affected with scrapie (Ersdal et al., 2005);
(Jeffrey et al., 2000); (Lezmi et al., 2001) and in experimentally BSE-infected sheep (Lezmi
et al., 2001); (Jeffrey et al., 2001). Interestingly, not all germinal centres were labelled for
PrPd; this partial absence of labelling in germinal centres (as in tonsils) was not observed in
samples from 13 natural scrapie-infected sheep in which all lymphoid germinal centres were
positively labelled for PrPd. This agreed with data describing an early and systematic immune
system involvement in lambs affected with scrapie (Andreoletti et al., 2000) which was not a
Copyright @ Acta Biochimica Polonica, Paper in Press, No. 1273
- 6 -
feature of BSE agent infection in sheep during the first passage (Jeffrey et al., 2001; Martin et
al., 2005).
In our study, as opposed to previous published results, in both ARQ/ARQ sheep, PrPd was
detected by IHC in all motor nerves and associated with Schwann’s cells (figure 2d, e). PrPd
deposits were similarly detected in all other tissue samples containing peripheral nerves, most
notably in nerves in muscle samples. This observation was not reported in other studies with
sheep BSE (Foster et al., 2001; Jeffrey et al., 2001). However, we observed the same type of
deposits in two other sheep (ARQ/VRQ) naturally affected with scrapie (data not shown) and
two previous articles report similar data in sheep with natural scrapie (Archer et al., 2004);
(Groschup et al., 1999).
PrPd presence was also identified in striated muscles for both ARQ/ARQ sheep. These
deposits were associated with neuromuscular spindles that are highly innervated structures
made of groups of myocytes surrounded by a thin fibrous capsule (figure 2k, l) and are a
specialized subset of myocytes implicated in proprioception. In the tongue of sheep, the
accumulation of PrPd in these structures was less evident. Only one study reported the PrPd
presence in the muscle of sheep affected with scrapie using IHC and ELISA (Andreoletti et
al., 2004). Here, sampling and analysis of different muscles were not systematic and thus the
ELISA/IHC results were not correlated. However, the accumulation in muscle tissue of PrPd
in sheep affected with scrapie is not systematic (Andreoletti et al., 2004). Recently,
pathological prion protein was detected in muscles of hamsters and mice infected with rodentadapted
BSE or vCJD (Thomzig et al., 2006). Previously, other studies failed to detect prion
in nerves and muscles of BSE- or scrapie-infected sheep (Foster et al., 2001); (Hamir et al.,
2004) possibly relying on the use of different pre-treatments and antibodies.
In conclusion, we have shown that the inoculation of the BSE agent of French origin by
peripheral routes to Lacaune sheep lead to the development of the clinical disease only in
Copyright @ Acta Biochimica Polonica, Paper in Press, No. 1273
- 7 -
ARQ/ARQ sheep. The distribution of PrPd in ARQ/ARQ sheep infected with BSE was very
similar to that described in natural scrapie. Overall, we demonstrated for the first time the
presence of PrPd in muscles and nerves of sheep infected experimentally with BSE agent,
which stresses the potential risk for humans related to consumption of sheep products from
sheep naturally infected with BSE. ...
snip...end...TSS
http://www.actabp.pl/pdf/Preprint/20061273.pdf
TSS
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I found this excerpt tremendously informative. I understand firewall feeding firsthand, and let me add that this is still ongoing, never will stop because it is cost effective. I related to the last excerpt personally...I am no longer in the biz, but we can all never really let things go and leave them behind.

Sent: Thursday, November 18, 2010 1:44 PM
Subject: UNITED STATES OF AMERICA VS GALEN J. NIEHUES FAKED MAD COW FEED TEST ON 92 BSE INSPECTION REPORTS FOR APPROXIMATELY 100 CATTLE OPERATIONS
UNITED STATES OF AMERICA VS GALEN J. NIEHUES FAKED MAD COW FEED TEST ON 92 BSE INSPECTION REPORTS FOR APPROXIMATELY 100 CATTLE OPERATIONS
4:10-cr-03119-RGK -CRZ Doc # 1 Filed: 11/16/10 Page 1 of 4 - Page ID #1
FILED
U.S. DISTRICT COURT
DISTRICT OF NEBRASKA
10 NOV 16 PM 4:16
OFFICE OF THE CLERK
IN THE UNITED STATES DISTRICT COURT
FOR THE DISTRICT OF NEBRASKA
UNITED STATES OF AMERICA,
Plaintiff,
vs.
GALEN J. NIEHUES,
Defendant.
INDICTMENT
18 U.S.C. § 1001
18 U.S.C. § 1341
The Grand Jury charges that:
INTRODUCTION
At all times material to this Indictment:
I. The U.S. Food and Drug Administration (FDA) was an agency within the U.S. Department of Health and Human Services, a part of the executive branch of the Government of the United States.
2. The FDA provided $250,000.00 in grant funds annually to the Nebraska Department of Agriculture (NDA) to perform inspections at cattle producing sites within the state to detect and identify the possible existence of Bovine Spongiform Encephalopathy (BSE), also known as "Mad Cow Disease".
3. The FDA published a final regulation designed to prevent the spread ofBSE through animal feed. The rule prohibited use of most mammalian protein, such as meat meal, bone meal, and hydrolyzed hair, in the manufacture of animal feeds given to cows, sheep and goats. The regulation also required process and control systems to ensure that feed for these animals does not contain prohibited mammalian tissue.
4. Defendant Galen J. Niehues was employed by NDA between approximately July 1, 2009 and March 31, 2010. Niehues' job duties included identifying cattle producers, performing on
-1-
4:10-cr-03119-RGK -CRZ Doc # 1 Filed: 11/16/10 Page 2 of 4 - Page ID # 2
site inspections of the farm site and cattle operations, interviewing cattle producers, taking samples of cattle feed and submitting the feed samples for laboratory analysis for the possible presence of BSE contaminants, and completing reports to document his on-site inspections and feed sample collection.
5. Between approximately July 1,2009 and March 31, 2010, Defendant Galen J. Niehues submitted reports he had completed and feed samples to NDA, for approximately 100 cattle operations within Nebraska as well as travel, per diem, lodging and miscellaneous expenses, by delivering-and placing the reports and related documents and forms to post offices in Lexington, Nebraska and Cozad, Nebraska, to be sent or delivered by the postal service to NDA in Lincoln, Nebraska, and knowingly causing the reports, documents and forms to be delivered by mail.
6. During the time of his employment, Defendant Galen J. Niehues was paid a total of $35,409.65 by NDA in salary and benefits.
7. Title 18 USC §IOOI (False Statement) prohibits anyone, in any matter, within the executive branch of the government of the United States, from making or using any false writing or document, knowing the same to contain any materially false, fictitious, or fraudulent statement or entry.
8. Title 18 USC § 1341 (Mail Fraud) prohibits anyone, having devised or intended to devise a scheme or artifice to defraud, or for obtaining money or property by means of materially false pretenses, representations, or promises, for the purpose of executing such scheme or artifice or attempting to do so, from placing in any post office or authorized depository for mail, any written matter or thing to be sent or delivered by the postal service, or knowingly causing any such matter or thing to be delivered by mail.
-2-
4:10-cr-03119-RGK -CRZ Doc # 1 Filed: 11/16/10 Page 3 of 4 - Page ID # 3
COUNT 1 - FALSESTATEMENT
9. Paragraphs 1 - 8 of this Indictment are realleged and incorporated as fully set forth herein.
10. On or about September 10, 2009, in the District of Nebraska, Defendant Galen Niehues, did knowingly and intentionally make, in a matter within the jurisdiction of the U.S. Department of Health and Human Services, a false BSE report, #EIR 3007935397, knowing the same to contain a materially false and fictitious statement in that the information and data contained in the reporthe prepared and submitted to NDA represented he had made contact with and interviewed the cattle owner, inspected the cattle owner's premises, and collected a sample of cattle feed, when in fact, Defendant Niehues, at the time he submitted the report on or about September 10,2009, then and there knew he had not made contact nor interviewed the owner or anyone associated with the cattle operation, nor had he obtained a sample of feed from the cattle operation as he represented in his report.
In violation of Title 18 United States Code §1001
COUNT II - MAIL FRAUD
11. Paragraphs 1 - 8 of this Indictment are realleged and incorporated as fully set forth herein.
12. Between approximately July 1, 2009, and March 31, 20 10, in the District of Nebraska, and elsewhere, Defendant Galen J. Niehues, devised and intended to devise a scheme and artifice to defraud, and to obtain money and property by means of material and false pretenses and representations.
13. It was part of the scheme and artifice to defraud, that in connection with his job duties and responsibilities with NDA, Defendant Galen 1. Niehues completed and submitted
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4:10-cr-03119-RGK -CRZ Doc # 1 Filed: 11/16/10 Page 4 of 4 Page ID # 4
approximately 92 BSE inspection reports and related documents which he knew contained materially false, fictitious and fraudulent statements. Specifically, the inspection reports were materially false, in that, they represented that the defendant had made contact with and interviewed the cattle owners, inspected the cattle owners' premises, and collected samples of cattle feed, when, in fact, he had not.
14. On or about September 11, 2009, Defendant Galen J. Niehues, as part of his scheme and artifice to defraud, and for the purpose of executing such scheme and artifice, and attempting to do so, placed copies of fabricated BSE reports and related documents in a post office and authorized depository for mail matter, in Cozad, Nebraska, to be sent and delivered by the postal service to NDJ\. in Lincoln, Nebraska, knowing such reports and related documents were to be delivered by mail.
In violation of Title 18 United States Code §1341.
The United States of America requests that trial of this case be held at Lincoln, Nebraska, pursuant to the rules of this Court.
WILLIAM W. MICKLE, II Assistant United States Attorney
FILED U.S. DISTRICT COURT DISTRICT OF NEBRASKA 10 NOV 16 PM 4:17 OFFICE OF THE CLERK
-4-...END...TSS
please see more here, with other bungled and blundered mad cow testing done ;
Wednesday, November 17, 2010
MAD COW TESTING FAKED IN USA BY Nebraska INSPECTOR Senator Mike Johanns STATE
http://madcowtesting.blogspot.com/2010/11/mad-cow-testing-faked-in-usa-by.html
Tuesday, November 02, 2010
IN CONFIDENCE
The information contained herein should not be disseminated further except on the basis of "NEED TO KNOW".
BSE - ATYPICAL LESION DISTRIBUTION (RBSE 92-21367) statutory (obex only) diagnostic criteria CVL 1992
http://bse-atypical.blogspot.com/2010/11/bse-atypical-lesion-distribution-rbse.html
Thursday, November 18, 2010
Increased susceptibility of human-PrP transgenic mice to bovine spongiform encephalopathy following passage in sheep
http://bse-atypical.blogspot.com/2010/11/increased-susceptibility-of-human-prp.html
Seven main threats for the future linked to prions
The NeuroPrion network has identified seven main threats for the future linked to prions.
First threat
The TSE road map defining the evolution of European policy for protection against prion diseases is based on a certain numbers of hypotheses some of which may turn out to be erroneous. In particular, a form of BSE (called atypical Bovine Spongiform Encephalopathy), recently identified by systematic testing in aged cattle without clinical signs, may be the origin of classical BSE and thus potentially constitute a reservoir, which may be impossible to eradicate if a sporadic origin is confirmed.
*** Also, a link is suspected between atypical BSE and some apparently sporadic cases of Creutzfeldt-Jakob disease in humans. These atypical BSE cases constitute an unforeseen first threat that could sharply modify the European approach to prion diseases.
Second threat
In small ruminants, a new atypical form of scrapie currently represents up to 50% of detected cases and even involves sheep selected for resistance to classical scrapie. The consequences for animal and human health are still unknown and there may be a potential connection with atypical BSE. These atypical scrapie cases constitute a second threat not envisioned previously which could deeply modify the European approach to prion diseases.
Third threat
The species barrier between human and cattle might be weaker than previously expected and the risk of transmission of prion diseases between different species has been notoriously unpredictable. The emergence of new atypical strains in cattle and sheep together with the spread of chronic wasting disease in cervids renders the understanding of the species barrier critical. This constitutes a third threat not properly envisioned previously that could deeply modify the European approach to prion diseases.
Fourth threat
Prion infectivity has now been detected in blood, urine and milk and this has potential consequences on risk assessments for the environment and food as well as for contamination of surfaces including medical instruments. Furthermore the procedures recommended for decontamination of MBM (Meat and Bone Meal), which are based on older methodologies not designed for this purpose, have turned out to be of very limited efficacy and compromise current policies concerning the reuse of these high value protein supplements (cross-contamination of feed circuits are difficult to control). It should be noted that the destruction or very limited use of MBM is estimated to still cost 1 billion euros per year to the European economy,
whereas other countries, including the US,
Brazil, and Argentine do not have these constraints.
However, many uncertainties remain concerning the guarantees that can be reasonably provided for food and feed safety and scientific knowledge about the causative agents (prions) will continue to evolve. This decontamination and environmental issue is a fourth threat that could modify deeply the European approach to prion diseases.
Fifth threat The precise nature of prions remains elusive. Very recent data indicate that abnormal prion protein (PrPTSE) can be generated from the brains of normal animals, and under some conditions (including contaminated waste water) PrPTSE can be destroyed whereas the BSE infectious titre remains almost unchanged, a finding that underlines the possibility of having BSE without any detectable diagnostic marker. These are just two areas of our incomplete knowledge of the fundamental biology of prions which constitute a fifth threat to the European approach to prion diseases.
Sixth threat The absence of common methods and standardisation in the evaluation of multiple in vivo models with different prion strains and different transgenic mice expressing PrP from different species (different genotypes of cattle, sheep, cervids, etc) renders a complete and comprehensive analysis of all the data generated by the different scientific groups almost impossible. This deeply impairs risk assessment. Moreover, the possibility of generating PrPTSE de novo with new powerful techniques has raised serious questions about their appropriateness for use as blood screening tests. The confusion about an incorrect interpretation of positive results obtained by these methods constitutes a sixth threat to European approach to prion diseases.
Seventh Threat The detection of new or re-emerging prion diseases in animals or humans which could lead to a new crisis in consumer confidence over the relaxation of precautionary measures and surveillance programmes constitutes a seventh threat that could modify the European approach to prion diseases.
http://www.neuroprion.org/en/np-neuroprion.html
Thursday, August 12, 2010
Seven main threats for the future linked to prions
http://prionpathy.blogspot.com/2010/08/seven-main-threats-for-future-linked-to.html
http://prionpathy.blogspot.com/
ALABAMA MAD COW g-h-BSEalabama
In this study, we identified a novel mutation in the bovine prion protein gene (Prnp), called E211K, of a confirmed BSE positive cow from Alabama, United States of America. This mutation is identical to the E200K pathogenic mutation found in humans with a genetic form of CJD. This finding represents the first report of a confirmed case of BSE with a potential pathogenic mutation within the bovine Prnp gene. We hypothesize that the bovine Prnp E211K mutation most likely has caused BSE in "the approximately 10-year-old cow" carrying the E221K mutation.
http://www.plospathogens.org/article/info%3Adoi%2F10.1371%2Fjournal.ppat.1000156
http://www.plospathogens.org/article/fetchObjectAttachment.action?uri=info%3Adoi%2F10.1371%2Fjournal.ppat.1000156&representation=PDF
Saturday, August 14, 2010
BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and VPSPr PRIONPATHY
(see mad cow feed in COMMERCE IN ALABAMA...TSS)
http://prionpathy.blogspot.com/2010/08/bse-case-associated-with-prion-protein.html
Wednesday, July 28, 2010
re-Freedom of Information Act Project Number 3625-32000-086-05, Study of Atypical BSE UPDATE July 28, 2010
http://bse-atypical.blogspot.com/2010/07/re-freedom-of-information-act-project.html
Monday, August 9, 2010
Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein or just more PRIONBALONEY ?
http://prionunitusaupdate2008.blogspot.com/2010/08/variably-protease-sensitive-prionopathy.html
PLEASE SEE the dramatic increase in sporadic CJD cases in documented BSE countries, then think, BSE can propagate as nvCJD and sporadic CJD in the lab ;
TOTAL CASES OF SPORADIC CJD (DEATHS) DEFINITE AND PROBABLE CASES
Australia Austria Canada France Germany Italy Netherlands Slovakia Spain Switzerland UK
http://www.eurocjd.ed.ac.uk/sporadic.htm
USA
5 Includes 16 cases in which the diagnosis is pending, and 18 inconclusive cases;
6 Includes 21 (19 from 2010) cases with type determination pending in which the diagnosis of vCJD has been excluded.
2010
PLEASE NOTE REFERENCE LINES 5. AND 6.
Monday, August 9, 2010
National Prion Disease Pathology Surveillance Center Cases Examined (July 31, 2010) Year Total Referrals2 Prion Disease Sporadic Familial Iatrogenic vCJD
1996 & earlier 51 33 28 5 0 0
1997 114 68 59 9 0 0
1998 88 52 44 7 1 0
1999 120 72 64 8 0 0
2000 146 103 89 14 0 0
2001 209 119 109 10 0 0
2002 248 149 125 22 2 0
2003 274 176 137 39 0 0
2004 325 186 164 21 0 1(3)
2005 344 194 157 36 1 0
2006 383 197 166 29 0 2(4)
2007 377 214 187 27 0 0
2008 394 231 204 25 0 0
2009 425 259 216 43 0 0
2010 204 124 85 20 0 0
TOTAL 3702(5) 2177(6) 1834 315 4 3
1 Listed based on the year of death or, if not available, on year of referral;
2 Cases with suspected prion disease for which brain tissue and/or blood (in familial cases) were submitted;
3 Disease acquired in the United Kingdom;
4 Disease was acquired in the United Kingdom in one case and in Saudi Arabia in the other case;
5 Includes 16 cases in which the diagnosis is pending, and 18 inconclusive cases;
6 Includes 21 (19 from 2010) cases with type determination pending in which the diagnosis of vCJD has been excluded.
http://www.cjdsurveillance.com/pdf/case-table.pdf
Monday, August 9, 2010
National Prion Disease Pathology Surveillance Center Cases Examined (July 31, 2010)
(please watch and listen to the video and the scientist speaking about atypical BSE and sporadic CJD and listen to Professor Aguzzi)
http://prionunitusaupdate2008.blogspot.com/2010/08/national-prion-disease-pathology.html
Atypical BSE in Cattle
BSE has been linked to the human disease variant Creutzfeldt Jakob Disease (vCJD). The known exposure pathways for humans contracting vCJD are through the consumption of beef and beef products contaminated by the BSE agent and through blood transfusions. However, recent scientific evidence suggests that the BSE agent may play a role in the development of other forms of human prion diseases as well. These studies suggest that classical type of BSE may cause type 2 sporadic CJD and that H-type atypical BSE is connected with a familial form of CJD.
To date the OIE/WAHO assumes that the human and animal health standards set out in the BSE chapter for classical BSE (C-Type) applies to all forms of BSE which include the H-type and L-type atypical forms. This assumption is scientifically not completely justified and accumulating evidence suggests that this may in fact not be the case. Molecular characterization and the spatial distribution pattern of histopathologic lesions and immunohistochemistry (IHC) signals are used to identify and characterize atypical BSE. Both the L-type and H-type atypical cases display significant differences in the conformation and spatial accumulation of the disease associated prion protein (PrPSc) in brains of afflicted cattle. Transmission studies in bovine transgenic and wild type mouse models support that the atypical BSE types might be unique strains because they have different incubation times and lesion profiles when compared to C-type BSE. When L-type BSE was inoculated into ovine transgenic mice and Syrian hamster the resulting molecular fingerprint had changed, either in the first or a subsequent passage, from L-type into C-type BSE. In addition, non-human primates are specifically susceptible for atypical BSE as demonstrated by an approximately 50% shortened incubation time for L-type BSE as compared to C-type. Considering the current scientific information available, it cannot be assumed that these different BSE types pose the same human health risks as C-type BSE or that these risks are mitigated by the same protective measures.
snip...see full text ;
http://www.prionetcanada.ca/detail.aspx?menu=5&dt=293380&app=93&cat1=387&tp=20&lk=no&cat2
14th ICID International Scientific Exchange Brochure -
Final Abstract Number: ISE.114
Session: International Scientific Exchange
Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009
T. Singeltary
Bacliff, TX, USA
Background:
An update on atypical BSE and other TSE in North America. Please remember, the typical U.K. c-BSE, the atypical l-BSE (BASE), and h-BSE have all been documented in North America, along with the typical scrapie's, and atypical Nor-98 Scrapie, and to date, 2 different strains of CWD, and also TME. All these TSE in different species have been rendered and fed to food producing animals for humans and animals in North America (TSE in cats and dogs ?), and that the trading of these TSEs via animals and products via the USA and Canada has been immense over the years, decades.
Methods:
12 years independent research of available data
Results:
I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc.
Conclusion:
I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. Restricting the reporting of CJD and or any human TSE is NOT scientific. Iatrogenic CJD knows NO age group, TSE knows no boundaries. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route.
page 114 ;
http://ww2.isid.org/Downloads/14th_ICID_ISE_Abstracts.pdf
The EMBO Journal (2002) 21, 6358 - 6366 doi:10.1093/emboj/cdf653
BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein
Emmanuel A. Asante1, Jacqueline M. Linehan1, Melanie Desbruslais1, Susan Joiner1, Ian Gowland1, Andrew L. Wood1, Julie Welch1, Andrew F. Hill1, Sarah E. Lloyd1, Jonathan D.F. Wadsworth1 and John Collinge1
1.MRC Prion Unit and Department of Neurodegenerative Disease, Institute of Neurology, University College, Queen Square, London WC1N 3BG, UK Correspondence to:
John Collinge, E-mail: j.collinge@prion.ucl.ac.uk
Received 1 August 2002; Accepted 17 October 2002; Revised 24 September 2002
--------------------------------------------------------------------------------
Abstract
Variant Creutzfeldt–Jakob disease (vCJD) has been recognized to date only in individuals homozygous for methionine at PRNP codon 129. Here we show that transgenic mice expressing human PrP methionine 129, inoculated with either bovine spongiform encephalopathy (BSE) or variant CJD prions, may develop the neuropathological and molecular phenotype of vCJD, consistent with these diseases being caused by the same prion strain. Surprisingly, however, BSE transmission to these transgenic mice, in addition to producing a vCJD-like phenotype, can also result in a distinct molecular phenotype that is indistinguishable from that of sporadic CJD with PrPSc type 2. These data suggest that more than one BSE-derived prion strain might infect humans; it is therefore possible that some patients with a phenotype consistent with sporadic CJD may have a disease arising from BSE exposure.
Keywords:BSE, Creutzfeldt–Jakob disease, prion, transgenic
http://www.nature.com/emboj/journal/v21/n23/abs/7594869a.html
BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein
Emmanuel A. Asante, Jacqueline M. Linehan, Melanie Desbruslais, Susan Joiner, Ian Gowland, Andrew L. Wood, Julie Welch, Andrew F. Hill, Sarah E. Lloyd, Jonathan D.F. Wadsworth, and John Collinge1 MRC Prion Unit and Department of Neurodegenerative Disease, Institute of Neurology, University College, Queen Square, London WC1N 3BG, UK 1Corresponding author e-mail: j.collinge@prion.ucl.ac.ukReceived August 1, 2002; Revised September 24, 2002; Accepted October 17, 2002.
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC136957/?tool=pubmed
TSS

The common denominator in all of these [transmissible mink encephalopathy - TME] outbreaks was either “cattle” or “unknown.” It was possible, of course, to imagine other scenarios, but Marsh believed he had at least strong circumstantial evidence that a TSE similar to mad cow disease already existed in U.S. cattle. “You can trace it back to feed real easy in mink,” Marsh said. “And then you’re left with the question, what was it in the feed that affected them? And what we find is it’s these downer cows that are the common link. You don’t have to be a genius to figure it out.”

Many thanks for taking the time to discuss this,
Also, the military apparently knew about this for at least a few years now to because they did some testing on patients in a veterans hospital a few years back that were originally diagnosed as having alzheimer’s disease, because the symptoms are nearly identical to those of alzheimers. If I recall correctly, somewhere around 6% of the patients that they thought had died of alzheimers had actually died of the human form of mad cow disease instead.
The reason that this seems to be such an unknown is that there is no set policy for testing alzheimers patients for mad cow disease at the time of their death. For those that don't know, the only definitive test for mad cow is to take a tissue sample from the affected areas and it is quite an expensive procedure. Until the government starts some sort of policy for testing alzheimers patients for mad cow disease at the time of their death, we may never know exactly how prevalent this problem really is in this country. thanks for the sharing / <a href="http://www.bigskyllc.com target=_blank" rel="follow">Church Construction </a>

I am starting to think that the cases in Miami, USA and Canada are related.
I hope the authorities are smart enough to have a biopsy done to check for a prion infection. Also there was a madman in Warwick ,RI last night that stripped naked, ran around making primal screams in the RI Mall, eventaully swam across the river, ran across 295 until getting caught and is now at a Mental hospital for observation. I think that's a rabies type symptom too.
I agree, I think all people who perish from Alzheimer's and Parkinson's disease should should be tested. It makes sense. Who knows, maybe Prion infections are responsible.